7Department of Community Health and Family Medicine, Mulago Hospital/Makerere University College of Health Sciences, Kampala, Uganda

8Ministry of Health Head Quarters, Kampala, Uganda

Correspondence to Dr Richard Idro; ridro1{at}gmail.com

Received 27 December 2012

Revised 5 April 2013

Accepted 8 April 2013

Published 3 May 2013

Abstract

Objectives Nodding syndrome is a devastating neurological disorder of uncertain aetiology affecting children in Africa. There is no
diagnostic test, and risk factors and symptoms that would allow early diagnosis are poorly documented. This study aimed to
describe the clinical, electrophysiological and brain imaging (MRI) features and complications of nodding syndrome in Ugandan
children.

Design Case series.

Participants 22 children with nodding syndrome brought to Mulago National Referral Hospital for assessment.

Outcome measures Clinical features, physical and functional disabilities, EEG and brain MRI findings and a staging system with a progressive
development of symptoms and complications.

Results The median age of symptom onset was 6 (range 4–10) years and median duration of symptoms was 8.5 (range 2–11) years. 16 of
22 families reported multiple affected children. Physical manifestations and complications included stunting, wasting, lip
changes and gross physical deformities. The bone age was delayed by 2 (range 1–6) years. There was peripheral muscle wasting
and progressive generalised wasting. Four children had nodding as the only seizure type; 18 in addition had myoclonic, absence
and/or generalised tonic–clonic seizures developing 1–3 years after the onset of illness. Psychiatric manifestations included
wandering, aggression, depression and disordered perception. Cognitive assessment in three children demonstrated profound
impairment. The EEG was abnormal in all, suggesting symptomatic generalised epilepsy in the majority. There were different
degrees of cortical and cerebellar atrophy on brain MRI, but no hippocampal changes. Five stages with worsening physical,
EEG and brain imaging features were identified: a prodrome, the development of head nodding and cognitive decline, other seizure
types, multiple complications and severe disability.

Conclusions Nodding syndrome is a neurological disorder that may be characterised as probably symptomatic generalised epilepsy. Clinical
manifestations and complications develop in stages which might be useful in defining treatment and rehabilitation. Studies
of risk factors, pathogenesis, management and outcome are urgently needed.